To date, the literature on PPGLs (both pheochromocytomas and paragangliomas have been described) associated with hypercortisolemia has primarily focused on tumors secreting ectopic ACTH and/or CRH, which predominantly present as overt Cushing syndrome (15, 16). This evidence concerns the gene CRH and Cushing syndrome due to macronodular adrenal hyperplasia.